Saturday, 5 January 2013

Too long to diagnose, almost too late

A Timeline of Julian's Health

Saturday, 21st June 2003
My Mum’s family had a birthday party at our local golf club for my Nanna’s birthday.  Up until this night, Julian had been a ‘normal’ little boy.  He had had the usual childhood ailments like tummy bugs and colds, and an intense dislike of mushrooms.   I remember Julian collapsing unconscious at Nigel’s feet.  He picked him up and we raced past family members (two of my Aunts are registered nurses, too!) into the office to call an ambulance.  While we waited, both my Aunts came in to check on him – it only took about 30 seconds for him to come to – and once the ambulance had arrived it was decided that he must have winded himself after jumping up on a chair and according to my nephew, hitting his chest on the table.  He was fine after that, played with the kids again and the night continued on.

After this night, he collapsed a couple of more times.  Once at his day care provider’s house, and as he was running down the hall with a toy, at the time no one was sure if he had tripped over and landed on the toy, causing him to wind himself.  The last time before he was diagnosed, he had been playing with friends at the end of the cul-de-sac (we were living in a secured townhouse complex) and he came up to me screaming and crying and collapsed at my feet, this time losing consciousness for about 3 minutes.  An ambulance was called – twice, as they didn’t turn up after almost 45 minutes – and he was taken to Logan Hospital, where a Registrar insisted that he had – again – winded himself.  This time, I refused to accept that response as I couldn’t understand how a 2 ½ year old could be ‘winded’ so frequently.  I demanded to see another doctor – or as I put it to the Registrar, someone who actually knows what they’re talking about! – and finally there was someone willing to listen to my concerns.  We were referred to the Paediatric Clinic at the Logan Hospital.

Tuesday, 2nd March 2004
After an EEG and CT Scan at Logan Hospital – despite my objections the Paediatrician was looking into Epilepsy– we were told that all the tests had come back negative, although they were still going to diagnosis him as Epileptic.  According to the Paediatrician, there are some forms of epilepsy that don’t show up during the tests.  I couldn’t accept the fact that despite a long family history of heart conditions in my family, the Paediatrician’s had not even ordered a simple ECG, and after a long, let’s call it a disagreement, they agreed to have an ECG done to – as they put it – “Put my mind at ease.”  Needless to say, it was ‘abnormal’, and the Paediatrician was of the opinion that it was something called ‘Long Q-T Syndrome’ and we were referred to the Cardiology Unit at the Mater Children’s Hospital.

Thursday, 4th March 2004
At this stage, I hadn’t had a very good return to work after maternity leave!  Days off for tests and specialist appointments had not put me in good stead with someone (not my boss, I should point out) I worked with, and he had decided that this was a good day to chip me about it.  Walking out of his office – fuming as I had offered to extend my leave for a few weeks but it was deemed unnecessary – I received a phone call from the Cardiology Unit at the Mater Children’s Hospital, asking us to take Julian in to the ‘Emergency Clinic’ that coming Saturday.    Nigel was in Mackay, visiting his brother, and wasn’t due back for another week, so I called him, let him know about the appointment and he drove back that night.

Saturday, 6th March 2004
Mum and Dad looked after our son Ayan, while we took Julian to the appointment that Saturday.  We were the only ones in the waiting room.  Five minutes of asking our family history and a quick check up of Julian was all it took for Dr. Cameron Ward to give us a tentative diagnosis of Idiopathic Pulmonary Arterial Hypertension – or as it was then known as, Primary Pulmonary Hypertension – and to tell us that he was hoping he was wrong. 

He had an ECG and Dr. Ward did an Echocardiogram; I think it took about 20 minutes.  The longest and most awful silence of my life as there was not a word spoken, except to ask Julian to lie on his side, back, etc.  When it was done, Dr. Ward had a quick word with his nurse – Julie Purdie – and she took Jules into the Starlight Room so we could talk.

Dr. Ward explained that he was confident Julian had Primary Pulmonary Hypertension, and said that he would have to have a cardiac catheterisation to establish pressure and finalise the diagnosis.  After this, medication and treatment could start.  They would contact us with a date, but it would need to be as soon as possible, given that Julian was already collapsing, which meant that the condition had progressed to a point where heart failure was possible.  We were told that this condition had a mortality rate of 2 – 5 years, and without treatment, Julian wouldn’t survive. 

Tuesday, 16th March 2004
Julian was admitted to the Children’s Ward at The Prince Charles Hospital on the Monday, and had the Cardiac Catheterisation on the Tuesday.  Both my parents and Nigel’s parents came in and waited with us.  We had been told that there was a risk Julian would not survive the procedure, so it was a huge comfort to us to have their support.

He came through it okay, although he did have hallucinations coming out of the anesthetic and I went on his bed with him to be taken up to the Ward, where Nigel, my Mum and I took turns walking the floor with him as he was extremely hysteric for hours.

Later that afternoon, we met with Julian’s new Pulmonary Hypertension specialist, Keith McNeill.  He explained that the diagnosis of PPH had been confirmed, and that they would start him on Warfarin (which Julian absolutely refused to take dissolved in a syringe, and at almost 3 ½ years old, quickly learned to swallow tablets instead) and Digoxin immediately.  Paperwork had to be submitted before he could start on something called ‘Bosentan’ as it was still a prescription that was under Federal approval.

We left the hospital a few days later, both relieved and lost in grief from the confirmation of Primary Pulmonary Hypertension.

Once he started on the Bosentan, Julian’s health improved dramatically and we began to accept this huge change to our life. 

January 2005

I’m not sure of the exact date, however I do remember that my Mum had to come in to hospital and relieve me.  I was huge with our daughter (our third child) and sleeping on the sofa/beds they had for parents was almost impossible with my stomach hanging over the side!  Not to mention Julian did not like being in hospital, and had pushed me to my emotional limits. 

Julian had been admitted to start a new medication – Sildenafil – and was under observation to make sure his blood pressure wasn’t affected.  He came home after three days, and improved immensely over the next few months.

We had moved to Hervey Bay in March of 2008, and Dr. Scott Fox took over as Julian’s cardiologist.  He was the regional children’s cardiologist, and seeing him took the pressure off returning to Brisbane every 3 months to see Dr. Ward.  We still had to come back to see Dr. McNeill though. 

Our son, Boehm (Boey) was born in December of this year as well.  It was a huge year!

In early 2010, a Pulmonary Hypertension clinic was started at the Mater Children’s Hospital, with a team of specialists comprising of Dr. Cameron Ward, Dr. Keith McNeill, Dr. Scott Fox, a Paediatric Cardiac Nurse, an Occupational Therapist, a Psychologist, a Physiotherapist and rotating Cardiac Fellows.  Nigel and I also made the decision to move again, this time to Tamborine Mountain, a move which has been one of the best decisions of our lives.

Julian’s health started to deteriorate this year, and continued to do so through 2011.

May 2011
Due to the deterioration of Julian’s health, the decision was made to create a hole in Julian’s heart (essentially creating an ASD – Atrial Septal Defect) and putting in a stent to keep it open.  The idea of this is to keep the pressure down in the heart, and prevent a collapse and possible heart failure by allowing blue (de-oxygenated) to leak from the right side of the heart to the left.  Normally, this would be creating problems in itself, but for Julian it was meant to prevent his condition from deteriorating further.

A date was made for the July school holidays, but Julian came down with a bad cold and it was postponed until August.

Monday 1st – Sunday 7th August 2011
Julian was admitted to the Cardiac Ward at the Mater Children’s Hospital on Monday 1st August in preparation for the procedure which was booked for Wednesday 3rd.  It was your usual check-in, with lunch at the Sushi Train (highly recommended!), and wonderful Starlight volunteers to keep the kids occupied after dinner.

Julian fasted all day on Wednesday, and was booked in for his procedure at 4pm.   Unfortunately, a little boy in the same room as Jules - just 2 years old – ended up in a coma after his procedure and Julian’s doctor’s made the decision to postpone his procedure to the next day when they were refreshed and not so exhausted after their fight to save this little boy.  At the time of Julian’s release, little Jackson was still in a coma, and unfortunately, we never did find out how things went for him and his family.  The nurses on the Cardiac Ward gave Julian a gift from a charity called ‘Precious Hearts’, which was a bag of puzzles, games, etc, as a reward for doing so well and keeping another little boy occupied and happy until his surgery.

Julian’s was first off the rank on Thursday 4th August, with Nigel walking into the operating theatre with him after I gave my boy and huge hug and a kiss.  We spent a nervous 3 hours waiting to be called to the Recovery Room, and took a little advantage of the relief we felt to take a few videos of the hilarity of Julian coming out of anesthetic.  No hallucinations this time, but goodness, the things he was saying – we kept giggling and even the nurse cracked a smile.

He was released from hospital on Saturday 6th, although we had to return on the Sunday for another echo just to check that the stent was still functioning well.

It took longer for Julian to recover from this procedure than we all thought it would, and it took even longer to see the positive effects we were all hoping would be close to immediate.  There was talk that he would have to try a different treatment, leaving us with very little options in the future, but thankfully he eventually started to show an improvement, and continued to do so until he stabilised.

To Date
Julian’s health has been stable for quite some time now, and the Pulmonary Hypertension Clinics every 3 months are a great time to catch up with the other 3 kids in Queensland with Idiopathic Pulmonary Arterial Hypertension.  He has recently gone on a medication called ‘Sandimigran’ for the migraines he was suffering on a weekly basis.  Fingers crossed, he has only had 2 since he started it, and although he still has headaches a few times a week, they rarely blow out to a migraine now. 

He has good days and bad days.  Today is a good day.


  1. This will be such a great resource for anyone that googles his condition! Well done and well written!

  2. Thank you, Annet. It's been difficult getting this blog started on an emotional level, but I'm very glad I did! Over the next couple of weeks, I'm going to put up some more information about Julian's condition, and the symptoms that are linked to it. Hopefully it will be easier to understand what's going on with Julian and other kids like him.